小儿再生障碍性贫血的诊断及鉴别诊断

目的提高对小儿再生障碍性贫血(AA)诊断及鉴别诊断的认识。方法对30例CAA、17例骨髓增生异常综合征(MDS)及4例小儿急性淋巴细胞白血病前期(pre-ALL)做血象、骨髓象及骨髓活检分析。结果CAA组与MDS组血象单核细胞及血小板计数间比较(P<0·05),有显著性差异。CAA组骨髓增生低下,原始+早幼粒细胞、原始+早幼红细胞、巨核细胞明显少于MDS,各组间比较(P<0·01),有显著差异性,并见MDS各系病态造血。骨髓活检CAA组96%以上造血组织明显减少、脂肪组织明显增多,巨核细胞缺如或减少(均<2个/片),检出率9·7%,纤维组织增生检出率16·3%。MDS组70%增生良好,近60%检出红系同一阶段发育幼红细胞岛,近90%检出幼稚前体细胞异常定位(ALIP),全部病例见纤维组织增生和小巨核细胞等异常。4例pre-ALL示三系细胞减少,骨髓增生减低,巨核细胞0~6个/片,活检示脂肪组织增多,造血组织明显减少,亦可见病态造血,1~4周内转变为急性淋巴细胞白血病(ALL)。结论CAA患儿外周血细胞减少,骨髓造血功能衰竭,无病态造血。MDS外周血单核细胞增多,骨髓增生良好,具有多系病态造血;ALIP、巨核细胞形态异常及红系同一阶段发育幼红细胞岛伴纤维组织增生是其特征。pre-ALL具有CAA和MDS的临床及实验室特点,但在短期内转变为ALL又与之不同。

Diagnosis and differential diagnosis of aplastic anemia in children

Objective To improve diagnosis and differential diagnosis of aplastic anemia in children. Methods The examination of the peripheral blood smear, the bone marrow fragment and the bone marrow biopsy in 30 cases of the chronic aplastic anemia (CAA), 17 cases of the myelodysplastic syndromes (MDS) and 4 cases of the preceding acute lymphatic leukemia (pre-ALL). Results There are significantly differences in monocytes and platelet countin peripheral blood between the CAA and the MDS. The bone marrow fragments show a reduction of cellularity in the CAA. The counts of myeloblasts, promyelocytes, proerythroblast, basophilic erythroblast and megakaryocytes in the bone marrow of the CAA are significantly lower than that of the MDS, but lymphocyte count is higher than that of the MDS. The bone marrow in the MDS shows morphological abnormalities. The hemotopoietic tissue in the bone marrow decreases obviously in more than 96% of the children with CAA. The fat tissue in bone marrow is obviously increased. There is a reduction or deficiency (<2 cell/piece) of megakaryocytes in 9.7% of the children with CAA. Fibrin tissue increases in bone marrow in 16.3% of the children with CAA. The bone marrow is hypercellular in 70% of the MDS. 60% of bone marrow show the erythroblast islets inwhich the erythroblasts are in the same developed stage, 90% of bone marrow show abnormal localization of precursor cell (ALIP) and 100%show small megakaryocytes and an increase in the fibrin tissue in the MDS. In 4 children with pre-ALL, there are the decrease of all blood cell counts in the peripheral blood,reduction of cellularity and megakaryocytes (0～6cell/piece) in bone marrow, the decrease of the hemotopoietic tissue and the increase of the fat tissue in bone marrow biopsy. The bone marrow in the pre-ALL shows also the morphological abnormalities. The children with pre-ALL convert to ALL in 1～4 weeks. Conclusion There are a decrease of all blood cell counts in the peripheral blood and a reduction of cellularity in bone marrow, but there is no the morphological abnormalities in the children with CAA. Monocytes count in peripheral blood,fibrin tissue and cellularity in bone marrow all increase, the morphological abnormalities and ALIP appear in the children with MDS. The clinical ,blood and marrow features of the pre-ALL are similar to that of the CAA and the MDS,but the children with pre-ALL convert to ALL in short time.